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Effect of Copper on l-Cysteine/l-Cystine Influx in Normal Human Erythrocytes and Erythrocytes of Wilson’s Disease

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Abstract

Wilson’s disease is a disease of abnormal copper metabolism in which free serum copper level is raised. The objective of the study was to determine, whether in Wilson disease, l-cysteine/l-cystine influx into RBC was decreased or not and the specific amino acid transporter affected by copper in normal human RBC. For l-cysteine/l-cystine influx, ten untreated cases, ten treated cases and ten age and sex matched healthy controls were recruited. To study the effect of copper on l-cysteine/l-cystine influx in RBC, 15 healthy subjects were selected. RBC GSH and l-cysteine/l-cystine influx were estimated by Beautler’s and Yildiz’s method respectively. In untreated cases, l-cysteine/l-cystine influx and erythrocyte GSH level were decreased showing that elevated level of free copper in serum or media decreased l-cysteine/l-cystine influx in human RBC. Copper treatment inhibited L amino acid transporter in normal RBC specifically.

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Acknowledgments

The authors wish to acknowledge all the staff of the Department of Biochemistry, Calcutta National Medical College.

Author’s Contribution

Dr. Nabarun Mandal and Dr. Debojyoti Bhattacharjee developed the study protocol and were involved in patient screening and enrolement. Dr. Gorachand Bhattacharya conceptualised the study and participated in the development of the project. Dr. Chandan Sarkar and Dr. Jayanta Kumar Rout were involved in the measurement of parameters. Dr. Anindya Dasgupta contributed to data analysis. Dr. Prasanta Kumar Gangopadhyaya participated as an expert in diagnosis, treatment and follow up of cases.

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Correspondence to Debojyoti Bhattacharjee.

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All the authors involved in this study declares that they have no conflict of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Mandal, N., Bhattacharjee, D., Rout, J.K. et al. Effect of Copper on l-Cysteine/l-Cystine Influx in Normal Human Erythrocytes and Erythrocytes of Wilson’s Disease. Ind J Clin Biochem 31, 468–472 (2016). https://doi.org/10.1007/s12291-016-0555-z

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  • DOI: https://doi.org/10.1007/s12291-016-0555-z

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